PoPH is a subset of pulmonary arterial hypertension (PAH), linked with increased blood pressure in the portal vein, often due to cirrhosis.

Patients with the condition are often only diagnosed as part of an assessment for liver transplantation; however, severe PAH is a contraindication for liver transplant due to poor post-operative prognosis.

As lead investigator, Olivier Sitbon, Professor of Respiratory Medicine at the South Paris University, explains: "The findings of PORTICO are relevant because if patients with PoPH can be treated to successfully lower pulmonary vascular pressure and resistance, more patients may be eligible for liver transplant as they will potentially have a better prognosis for this surgery.

“The fact that the hepatic safety profile of macitentan in patients with PoPH was consistent with that observed in previous trials is particularly reassuring, as PoPH patients are typically excluded from PAH clinical trials on safety grounds.”

Opsumit is an orally active endothelin receptor antagonist (ERA) currently approved in the US and Europe for the treatment of PAH.